Case Report/Case Series |

Autoantibodies in Sporadic Creutzfeldt-Jakob Disease

Heather Angus-Leppan, MD, FRCP1; Peter Rudge, FRCP2; Simon Mead, PhD, FRCP2; John Collinge, MD, FRCP, FRS2; Angela Vincent, FRCPath, FRS3
[+] Author Affiliations
1Clinical Neurosciences, Royal Free London NHS Foundation Trust and Barnet Chase Farm Hospitals NHS Trust, London, England
2MRC Prion Unit and National Prion Clinic, National Hospital for Neurology and Neurosurgery, London, England
3Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, England
JAMA Neurol. 2013;70(7):919-922. doi:10.1001/jamaneurol.2013.2077.
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Importance  The diagnosis of autoimmune and neurodegenerative conditions can be unclear. Treatments such as removing the associated tumor, if present, and immunosuppression can halt or often reverse the progression of autoimmune conditions, but there is no curative treatment for neurodegenerative conditions. The presence of autoantibodies can sometimes be misleading. This report illustrates potential difficulties in differentiating autoimmune encephalopathies from sporadic Creutzfeldt-Jakob disease.

Observations  In a clinical follow-up of an older man with rapidly evolving encephalopathy at a neuroscience center, unsuccessful treatment with immunosuppression based on the incorrect presumptive diagnosis of Morvan syndrome was followed by the correct histological diagnosis of sporadic Creutzfeldt-Jakob disease.

Conclusions and Relevance  Autoimmune encephalopathies raise important treatment options and potential for recovery. However, since neuronal antibodies may be positive in prion disease, interpretation can be complex and must be rooted in the clinical picture.

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Antibody Findings and Histopathological Analysis

A, Serum voltage-gated potassium channel complex antibodies (VGKC Ab) and glycine receptor antibodies (GlyR Ab) during the course of the illness. The cutoff values for healthy subjects are indicated by a dashed line. *Negative cerebrospinal fluid titers of each antibody. Treatment modality durations and a summary of symptom development are shown. IVIg indicates intravenous immunoglobulin. B, Typical spongiform change in frontal biopsy (hematoxylin-eosin, scale bar = 100 μm). C, Abnormal prion protein immunoreactivity in the biopsy specimen of the frontal brain showing synaptic distribution with ICSM35 monoclonal antibody (scale bar = 100 μm).

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Creutzfeldt-Jakob disease. JAMA Neurol 2013;70(12):1588-9.
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